Applications

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application

Abreviews

Notes

Sandwich ELISA

Use a concentration of 1 - 2 µg/ml. increasing amount of recombinant SOD1 captured by a rabbit antibody. ab62800 used as reporter antibody.

WB

Use a concentration of 0.01 - 0.03 µg/ml. Detects a band of approximately 18 kDa (predicted molecular weight: 16 kDa).

ELISA

1/8000. Peptide ELISA

ICC/IF

Use at an assay dependent concentration.

Target

FunctionDestroys radicals which are normally produced within the cells and which are toxic to biological systems.

Involvement in diseaseDefects in SOD1 are the cause of amyotrophic lateral sclerosis type 1 (ALS1) [MIM:105400]. ALS1 is a familial form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of cases leading to familial forms.

Post-translationalmodificationsUnlike wild-type protein, the pathogenic variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 are polyubiquitinated by RNF19A leading to their proteasomal degradation. The pathogenic variants ALS1 Arg-86 and Ala-94 are ubiquitinated by MARCH5 leading to their proteasomal degradation.The ditryptophan cross-link at Trp-33 is reponsible for the non-disulfide-linked homodimerization. Such modification might only occur in extreme conditions and additional experimental evidence is required.

ab62800 (1/200) staining Superoxide Dismutase 1 in HeLa cells (green). Cells were fixed in paraformaldehyde, permeabilised with 0.5%Triton X100/PBS and counterstained with DAPI in order to highlight the nucleus. Please refer to Abreview for further experimental details.